The Society for Vascular Surgery Clinical Practice Guidelines for the Management of Visceral Artery Aneurysms: Evidence Tables

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1 The Society for Vascular Surgery Clinical Practice Guidelines for the Management of Visceral Artery Aneurysms: Evidence Tables Renal Artery Aneurysms Methodological Study Population Interventions Outcomes Quality 1. Diagnosis and Evaluation Recommendation 1.1: In patients who are suspected to have RAA, we recommend computed tomographic angiography (CTA) as the diagnostic tool of choice (Grade 1B). Klausner, Ruptured and Nonruptured Zhang, Consecutive patients undergoing arterialphase contrastenhanced CT scan of the abdomen (N862) Open repair/nephrectomy Endo Surveillance Not applicable 1 CT angiography was the most frequently used imaging modality for the RAA diagnosis (58%), CT (without contrast) was the next most frequent (24%) RAA identified in 6 patients Some can only be displayed by post-processing techniques such as volume rendering and maximumintensity projection Multi- Institutional Review (VLFDC) / MODERATE consecutive /LOW Recommendation 1.2: In patients who are suspected to have RAA and have increased radiation exposure risks or renal insufficiency, we recommend non-contrast MRA (NC-MRA) to establish the diagnosis (Grade 1B). Technical remark: NC-MRA is best suited to children and women of childbearing potential or those who have contraindications to CT or MR contrast materials (i.e. pregnancy, renal insufficiency, or gadolinium contrast material allergy). Angeretti, comparison with contrast-enhanced MR angiography for N/A NC-MRA is a valid alternative to CE-MRA for the assessment of renal review by two

2 Study Population Interventions Outcomes Mohrs, patients with RVH (N63) Patients with suspected renovascular disease (N45) MRA of the renal arteries using a steady-state free precession (SSFP) technique in comparison with 1.0-molar contrastenhanced MRA in patients with suspected renal artery stenosis. Albert, (N75) Each patient underwent abdominal contrastenhanced CTA and abdominal unenhanced MRA using time-slip with balanced steady-state free precession arteries 99.4% of those detected by CE-MRA Sensitivity, specificity, and positive and negative predictive values of unenhanced MRA to detect renal artery stenoses greater than 50% were 75%, 99%, 75%, and 99%, respectively Unenhanced MRA correctly classified 17/23 renal arteries with > 50% stenosis and correctly classified 128/138 renal arteries as not having disease ( 50% stenosis) (sensitivity 74%, specificity 93%) Of the 16 misclassified arteries, only three = clinically relevant Methodological Quality independent radiologists diagnostic of consecutive patients Prospective international multi-center trial with two blind reviewers Recommendation 1.3: We recommend the use of catheter-based angiography for both preoperative planning and to better delineate distal renal artery branches that may be inadequately assessed on conventional cross sectional imaging (Grade 1C). Pfeiffer, Ruptured and Nonruptured (94) Open repair (in situ and ex vivo repair) Preoperative angiogram obligatory Review 2

3 Study Population Interventions Outcomes Endo, (N28) Rotational DSA images were obtained during a deep inspiratory pause after contrast material injection through the renal artery. Acquired data were transferred to an image workstation and reconstructed as 3-D images In all cases of aneurysm 3-D images were obviously more useful than 2- D DSA Methodological Quality case Liu, N/A N/A Catheter-based arteriogram = gold standard case 2. Size criteria and alternate indications for Recommendation 2.1: In patients with non-complicated RAA of acceptable risk, we suggest treatment for aneurysm size >3cm (Grade 2C). Klausner, Henriksson, Non-ruptured Surveillance (N88) Open surgical repair Endo Elective open repair (N15) Rupture (N4) Surveillance (N34) Tham, Non-ruptured Elective open repair (N14) Surveillance (N69) 3 No rupture in surveillance group (including 7 measuring >3cm without complication or rupture) No rupture (and null growth) in 34 patients followed for natural history No rupture (no reference to growth) in surveillance group or amongst autopsy patients Multi- Institutional Review review of 9500 angiographies / LOW review of 8525

4 Study Population Interventions Outcomes Martin, Henke, Chandra, Klausner, Wayne, Non-ruptured Ruptured and Nonruptured Non-ruptured Non-ruptured Elective open repair (N18) Nephrectomy (N4) Surveillance (N18, only 1 with imaging) Open Repair or Nephrectomy (N121) Surveillance (N47) Open repair (N9) Surveillance (N5) Open repair (N15) Surveillance (N25) Non-ruptured Surveillance 7 underwent open repair 4 No rupture in surveillance group No rupture in surveillance group No rupture in surveillance group No rupture; growth rate = / mm/y No rupture; Median annualized growth rate = 0.06 mm Methodological Quality angiographies + autopsy review Review and Autopsy Review / LOW Recommendation 2.2: We recommend emergent for any size renal artery aneurysm resulting in patient symptoms or rupture (Grade 1B) Henke, Ruptured and Nonruptured No mortality in ruptures Cochennec, Ruptured and Nonruptured Open Repair or Nephrectomy (N121) nephrectomy required for all cases of rupture; Surveillance (N47) Open repair/nephrectomy Endo 25% mortality in cases of rupture (1/4) by literature review Review AND

5 Study Population Interventions Outcomes Klausner Kok, Ruptured and Nonruptured Ruptured and Nonruptured Surveillance Open repair/nephrectomy Endo Surveillance Endo (embo with coils, N-butyl cyanoacrylate, thrombin injection), flow-diverting stent Open repair 5 0% mortality in cases of rupture (0/3) treated with nephrectomy (1) and embo (2) 3.8% mortality in cases of rupture (4/103) Methodological Quality Literature Multi- Institutional Review (VLFDC) / MODERATE Review + Systematic Review / LOW Pfeiffer, Ruptured and Nonruptured 25% mortality in cases of rupture (1/4) Recommendation 2.3: In patients of child-bearing potential with non-complicated RAA of acceptable risk, we suggest treatment for aneurysm size <3cm (Grade 2B). Cohen, Ruptured Nephrectomy No maternal or fetal mortality (Lit review identified 56% maternal and 86% fetal mortality) Hellmund, Ruptured Non-ruptured, complex RAA; sub-set treated exclusively for HTN Covered stent grafting Open repair (in situ autogenous repair and ex vivo repair) No maternal or fetal mortality (Lit review identified 35% maternal and 60% fetal mortality) Case Report and Literature Review (18 cases or gestational rupture) / LOW Case Report and Literature Recommendation 2.4: In patient with medically-refractory hypertension and functionally important renal artery stenosis, we suggest treatment for size <3cm (Grade 2C). Robinson, % improvement in HTN (9/11)

6 Study Population Interventions Outcomes Pfeiffer, Ruptured and Nonruptured; sub-set treated with HTN (N75) Martin, Henke, Chandra, Klausner, Non-ruptured; sub-set with HTN RVH (N9); Primary HTN (N24) Ruptured and Nonruptured Non-ruptured; sub-set with HTN underwent surgery (N7) Non-ruptured; sub-set with HTN (N14) Open repair (in situ and ex vivo repair) Open repair + nephrectomy Open Repair + Nephrectomy Open repair (in situ and ex vivo) Surveillance Open repair (in situ, ex vivo, nephrectomy) 47% improvement in HTN cure (N19/75) and improvement (N17/75) RVH Cure (2); improvement (5) Primary HTN Cure (2); improvement (4); no change (4) 60% (with follow-up) improvement in HTN Reduction in # of anti-hypertensive medications in hypertensive patients treated surgically No HTN benefit: cure (1); improvement (3); no change (9) Methodological Quality Klausner, English, Ruptured and Nonruptured; sub-set with medically refractory HTN (N=76) Non-ruptured; sub-set with HTN (89%) 25 patients had renal artery stenosis/atherosclerosis Open repair/nephrectomy Endo Open repair (in situ, ex vivo, nephrectomy) No HTN benefit: Cure (N24); Improvement (N20); no change (N32) essentially no change in mean BP or # of anti-hypertensive meds 75% with HTN benefit: Cure (N21%); Improved (54%) 3. Treatment options Recommendation 3.1: We suggest daily anti-platelet therapy (ie: Aspirin, 81mg) for patients with renal artery aneurysm (Grade 2C). Multi- Institutional Review (VLFDC) Review or prospectively maintained registry / HIGH 6

7 Study Population Interventions Outcomes Methodological Quality English, Non-ruptured; 25 patients had renal artery stenosis/atherosclerosis Open repair (in situ, ex vivo, nephrectomy) 8-11% incidence of renal thromboembolism Review or prospectively maintained registry / HIGH Recommendation 3.2: We suggest open surgical reconstructive techniques for the elective repair of most renal artery (Grade 2B). Bonardelli, Non-ruptured (N15) Open or video/lapassist 0% mortality (30d) Buck, Non-ruptured Open repair / nephrectomy (1627) VS. Endo (1082) 1% mortality (30d); 4.5% respiratory complications Comparative (Open v Endo) / LOW Duran, Non-ruptured (N80) Open repair (aneurysmorrhaphy) English, 2004 Non-ruptured (N62) Open repair (In 23 situ) Nephrectomy Foley, (abstract) Hislop, Klausner, Mixed (N12) N/R (N124 treated open) Non-ruptured (N15) Open repair (In situ, ex vivo and auto transplant) Nephrectomy Open repair Endo repair Open repair (In situ and ex vivo) 1.2% mortality (30d); 1.2% MI, 5% re- 1.6% mortality (30d); 3% SSI Review of Consecutive Registry / HIGH 0% mortality (30d) 3% mortality (30d) Review (State Database) / LOW 0% mortality (30d) 7

8 Study Population Interventions Outcomes Laser, Non-ruptured (N14) Open repair (Ex vivo) Marone, Pfeiffer, Robinson, Non-ruptured (N18) Ruptured and Nonruptured (N94) Non-ruptured, complex RAA (N24) Open repair (In situ) Open repair (In situ) Open repair (Complex in situ and ex vivo) 0% mortality (30d); 14% re; 7% end-organ infarction 0% mortality (30d); 5.6% end-organ infarction at longest f/u 1% mortality (30d); 1% MI; 4.2% re at longest f/u (mean 46mo) Methodological Quality Review / HIGH 0% mortality (30d) Recommendation 3.3: We suggest ex vivo repair and auto-transplantation for complex distal branch over nephrectomy when technically feasible (Grade 2B). Chandra, Non-ruptured Open repair (in situ and ex vivo) 0% mortality (last f/u mean 11.6mo), 0% re- Laser, Non-ruptured (N14) Open repair (Ex vivo) 0% mortality (30d); 14% re; 7% end-organ Review / HIGH Robinson, Murray, Gallagher, Non-ruptured, complex RAA (N24) Non-ruptured aneurysmal or occlusive disease (RVH) (N68) Non-ruptured (N7) Open repair (Complex in situ and ex vivo) Complex in situ and ex vivo repair Lap nephrectomy with ex vivo repair infarction 0% mortality (30d) 0% mortality; 90% primary patency; 94% stable to improved renal function; 0% mortality; No change in renal function 8

9 Study Population Interventions Outcomes Methodological Quality Recommendation 3.4: We suggest endovascular techniques for the elective repair of anatomically appropriate RAAs to include stent graft exclusion of main renal artery and embolization of distal and parenchymal (Grade 2B). Buck, Non-ruptured Open repair / nephrectomy (1627) VS. Endo (1082) (Coil or stent placement) Etezadi, Ruptured and Nonruptured (True and PSEUDO ) (N17) Coils + stent (6); coil embo (9); covered stent (2) 1.8% mortality (30d); 4.3% respiratory complications 0% mortality; 5.9% re-; 11.8% end-organ ischemia; Hislop, N/R (91 treated endo) Endo (N/R) 1% mortality; 0% respiratory complications Kok, Ghoneim, Sedat, Pseudo and True (>3 and <3cm in size) Ruptured and nonruptured (N6) Pseudoaneurysm following partial nephrectomy (N15) Non-ruptured (True) (N15) Endo (embo with coils, N-butyl cyanoacrylate, thrombin injection), flow-diverting stent Embo (coils) Embolization (coils) +/- adjuncts (ie: stents) + covered stent (3) Klein, Non-ruptured (12) Embo (conventional non-detachable Comparative (Open v Endo) / LOW Review (State Database) / LOW 0% mortality; 0% re- Review + systematic literature review) / LOW 2.2% re-; 2.2% DVT; 2.2% PE; 0% PES 6.7% end-organ infarction; 0% mortality (longest mean 52mo) 16.7% end-organ infarction; 8.3% re Review / MODERATE 9

10 Study Population Interventions Outcomes coils) or guglielmi detachable coils Ikeda, Non-ruptured (7) Coil Embolization 28.6% end-organ infarction; 28.6% coil migration; Lagana, Yasumoto, Ruptured and nonruptured; True and Pseudo (6) Non-ruptured (calcified and partially thrombosed excluded) (N8) Embolization (coils, adjuncts) and covered stent 16.7% mortality (1y); stent occlusion (x1) requiring thrombolysis Coil embolization 0% mortality mean 37mo); 37.5% re-; 25% end-organ infarction Methodological Quality Recommendation 3.5: We suggest consideration of laparoscopic and robotic techniques as an al alternative based on institutional resources and surgeon experience with minimally invasive techniques (Grade 2C). Luke, Non-ruptured, expanding, True RAA Robotic-assisted primary repair No mortality or major morbidity Case Report / LOW Giulianotti, Non-ruptured (N5) Robotic-assisted lap in situ repair 0% mortality; 20% re- case / Low Gheza, Non-ruptured Robotic-assisted in situ repair No mortality or major morbidity Case Report / LOW Samarasekera, Non-ruptured Robotic-assisted in situ repair No mortality or major morbidity Case Report / LOW 4. Screening Recommendation 4.1: We suggest screening female patients for fibromuscular dysplasia with a focused history and onetime axial imaging study (ie: CTA or MRA) to assess for cerebrovascular, mesenteric and iliac arterial dysplasia (Grade 2C). Olin, AHA recommends a focused vascular review of symptoms for all patients diagnosed with FMD, with an emphasis on quality of life-impairing symptoms like migraine headache, tinnitus and neck pain Additionally, one-time screening for occult aortic / arterial aneurysm in these patients is recommended Adapted from AHA Consensus Statement 10

11 Study Population Interventions Outcomes Methodological Quality 5. Follow-up and surveillance Recommendation 5.1: We suggest completion imaging following open surgical reconstruction for renal artery aneurysm, prior to hospital discharge, by way of arteriogram or ultrasound and long-term follow-up with surveillance imaging (Grade 2C) Robinson, Henke, English, Non-ruptured, complex RAA (N24) Ruptured and Nonruptured (N168) Non-ruptured (62) Pfeiffer, Ruptured and Nonruptured (94) Open repair (in situ autogenous repair and ex vivo repair) Open Repair + Nephrectomy Open repair (in situ, ex vivo, nephrectomy) Open repair (in situ and ex vivo repair) Among 18 patients who underwent duplex or contrasted imaging, one late failure of secondary patency was noted at 9 months in a patient who had undergone aneurysmorrhaphy and patch angioplasty (no re-) Among patients with an early successful repair, seven patients had later technical abnormalities recognized that required further Post-op assessment by angio/duplex intra-op revealed 5 technical problems resulting in intraop/immediate revision Completion duplex revealed one patient with stenosis requiring revision Review or prospectively maintained registry Recommendation 5.2: For patients managed non-operatively, we suggest annual surveillance imaging until two consecutive studies are stable; thereafter surveillance imaging may be extended to every 2-3 years (Grade 2B). Klausner, Surveillance (N88) Open surgical repair Endo No rupture in surveillance group; Aneurysm growth rate = cm/y, with no Multi- Institutional 11

12 Study Population Interventions Outcomes Klausner, Wayne, Non-ruptured Open repair (N15) Surveillance (N25) Non-ruptured Surveillance 7 underwent open repair difference between calcified and noncalcified No rupture; growth rate = / mm/y No rupture; Median annualized growth rate = 0.06 mm Methodological Quality Review (VLFDC) / MODERATE 12

13 Splenic Artery Aneurysms Population Interventions Outcomes Design 1. Diagnosis Recommendation 1.1: We recommend computerized tomographic angiography (CTA) as the initial diagnostic tool of choice for splenic artery. (Grade 1C) Saba, SAA CT imaging Imaging quality review Recommendation 1.2: In patients with suspected splenic artery and pre-existing renal insufficiency limiting the use of iodinated contrast material, we suggest magnetic resonance angiography (MRA) to establish diagnosis. (Grade 1C) Pilluel, non-ruptured SAA MRA vs. CT MRA quality similar to CT case Recommendation 1.3: We recommend using arteriography when non-invasive studies have not sufficiently demonstrated the status of relevant collateral blood flow and when endovascular is planned. (Grade 1B) Pilluel, non-ruptured SAA Angiography in selected patients Confirmed SAA in selected patients case Nosher, Visceral artery (VAA) Angiography, endovascular Collateral blood flow, techniques of embolization Case and review 2. Criteria for invasive Recommendation 2.1: We recommend emergent for ruptured splenic artery. (Grade 1A) Stanley, Ruptured Surgical repair 25% mortality case Lakin, Ruptured Surgical repair 2/7 mortality at 30 days case Recommendation 2.2: We recommend treatment of non-ruptured splenic artery pseudo of any size in patients of acceptable risk due to the possibility of rupture. (Grade 1B) Tulsyan, Observation, Embolization, surgical ligation 80% pseudo were symptomatic vs. 30% true case 13

14 Pitton, Embolization, surgical repair 14 Significantly higher rate of rupture at presentation for pseudo case Recommendation 2.3: We recommend treating non-ruptured splenic artery true of any size in women of childbearing age due to the risk of rupture. (Grade 1B) Dave, Observation, surgical repair Higher rate of rupture third trimester case Holdsworth, Ruptured SAA surgical repair, emergency fetal care Ruptured SAA leads to high maternal and fetal mortality Review Recommendation 2.4: We recommend treating non-ruptured splenic artery true more than 2 cm in size, with a demonstrable increase in size, or with associated symptoms in patients of acceptable risk due to the risk of rupture. (Grade 1C) Lakin, Non-ruptured SAA Observation, Embolization, Ligation 128 patients over 15 years case Tessier, 2003 Observation, Embolization, 217 patients Review 57 surgical repair Guo, SAA Endovascular repair 16 symptomatic patients vs. 71 asymptomatic patients; symptoms were associated with higher mortality case Recommendation 2.5: We suggest observation over repair for small (< 2 cm), stable asymptomatic splenic artery true, or those in patients with significant medical comorbidities or limited life expectancies. (Grade 2C) Lakin, Non-ruptured SAA Observation average growth rate 0.2 mm/year, no mortality case 3. Treatment options Recommendation 3.1: In patients with ruptured SAA discovered at laparotomy, we suggest treatment with ligation with or without splenectomy depending on the aneurysm location. (Grade 2B) Mattar, Ruptured Surgical repair 23 case experience case Abbas, Embolization, ligation 20 year experience case Recommendation 3.2: In patients with ruptured SAA diagnosed on preoperative imaging studies, we suggest treatment with open surgical or appropriate endovascular techniques based upon the patient s anatomy and underlying clinical condition. (Grade 2B)

15 Carroccio, Embolization, Ligation 17 patient experience case Recommendation 3.3: We suggest elective treatment of SAA using an endovascular approach if anatomically feasible. However, elective treatment may appropriately involve open surgical, endovascular or laparoscopic methods of depending on the patient s anatomy and underlying clinical condition. (Grade 2B) Lakin, Coil and glue embolization Low rate of complications, re in 49 patients case Yasumoto, Elective VAA coil embolization Low rate of re-, endorgan infarct in 15 patients case Dorigo, Non-ruptured endovascular treatment No mortality, 1/15 required re- case Pulli, Hogendoorn, Embolization, endovascular repair, surgery Embolization, endovascular repair, surgery, conservative management Variety of open techniques in 29 patients: resection with revascularization, splenectomy, ligation Endovascular repair is cost effective vs. open repair case Markov model Recommendation 3.4: When treating SAA, we suggest that the splenic artery does not routinely require preservation or revascularization. (Grade 2C) Chadha, Non-ruptured Embolization Review Pfister, Non-ruptured Open surgical repair 1/7 patients with end organ ischemia case Corey, SAA Endovascular therapy 4/32 developed end-organ infarction case Recommendation 3.5: When treating distal SAA adjacent to the hilum of the spleen, we suggest open surgical techniques including possible splenectomy as opposed to endovascular methods given concern regarding the possibility of end organ ischemia, including splenic infarction and / or pancreatitis. (Grade 2B) Marone, Non-ruptured Surgical repair 11/34 patients (32%) had distal SAA precluding splenic salvage case 15

16 Recommendation 3.6: In pregnant women with SAA, we recommend that treatment decisions be individualized and consider the potential morbidity to both the mother and fetus. (Ungraded best practice statement) Barrett, Ruptured primary repair, ligation >90 cases of ruptured SAA during pregnancy Review 4. Screening for concomitant Recommendation 4.1: We suggest screening of patients with splenic artery for other arterial. (Grade 2B) Abbas, Imaging case 3.3% other visceral 14.3% other non visceral 5. Follow-up and surveillance Recommendation 5.1: In patients in whom a splenic artery aneurysm is being followed with a non-operative / non-al approach, we suggest annual surveillance with CT scans to assess for growth in size. (Grade 2B) Al-Habbal, Imaging CT imaging is the best modality case Tessier, Imaging Growth of 0.06 cm/year case Recommendation 5.2: Following endovascular for splenic artery, we suggest periodic surveillance with appropriate imaging studies to assess for the possibility of endoleak or other continued aneurysm perfusion which could lead to a continued risk of aneurysm growth or rupture. (Grade 2B) Lakin, Embolization with coils and glue CT imaging appropriate but coil/glue artifact hinders endoleak detection case 16

17 Celiac Artery Aneurysm Population Interventions Outcome Design 1. Diagnosis Recommendation 1.1 We suggest computerized tomographic angiography (CTA) as the initial diagnostic tool of choice for celiac artery. (Grade 2B) Stone, Celiac artery review Vasconcelos, Sachdev, Saba, Celiac artery Celiac artery and SMA Splenic artery Abdominal imaging, open, endovascular and expectant management Abdominal imaging, open surgical management Abdominal imaging, open, endovascular and expectant management CT imaging 17 Diagnosis of celiac aneurysm is most commonly established by CT scan Imaging findings, open, surgical management Imaging findings, open, endovascular and expectant management Imaging characteristics of multi-detector CTA to assess visceral Case and review review review Recommendation 1.2 We suggest magnetic resonance angiography (MRA) in patients with suspected celiac AA and preexisting renal insufficiency limiting the use of iodinated contrast material. (Grade 2B) Pilluel, Visceral artery MRA and CTA imaging Good correlation between CTA and review Nosher, Visceral artery MRA and CTA imaging MRA Radiologic characteristics of visceral artery Review of imaging

18 Pilluel, Visceral artery MRA and CTA imaging Imaging quality of 3D contrast enhanced MRA Review of imaging Recommendation 1.3 We suggest arteriography when non-invasive studies have not sufficiently demonstrated the status of relevant collateral blood flow or when endovascular is planned. (Grade 2C) Vasconcelos, Stone, Nosher, Celiac artery Celiac artery Visceral artery Tulsyan, Visceral artery 2. Criteria for invasive Open surgical Abdominal imaging, open, endovascular and expectant management MRA and CT imaging, arteriography Endovascular management Operative outcome and planning Imaging findings, open, endovascular and expectant management Arteriography, endovascular Procedural outcome Case report and review review Case and review review. Recommendation 2.1 We recommend emergent for ruptured celiac artery. (Grade 1A) Shanley, Stone, Graham, Splenic, hepatic and celiac artery Celiac artery Celiac artery aneurysm Review of presentation, management and outcome Surgical management, observation Open surgical Rupture rate may approach 100% Surgical risks, outcome with observation, morbidity of rupture Operative outcome Review review case review 18

19 Guo, Carr, Visceral artery Visceral artery Open surgical, endovascular or conservative therapy Surgical management of rupture Symptomatic patients had a higher mortality rate and re- rate. Mortality of celiac aneurysm rupture review review approaches 100% Recommendation 2.2 We recommend treatment of non-ruptured celiac artery pseudo of any size in patients of acceptable risk due to the possibility of rupture. (Grade 1B) Vasconcelos, McMullan, Stone, Graham, Shukla, Celiac artery Celiac artery Celiac artery Celiac artery Visceral artery Open surgical Pseudo more likely to rupture Surgical Perioperative outcome Abdominal imaging, open, endovascular and expectant management Open surgical Outcome of intact and ruptured visceral artery ; open Imaging findings, open, endovascular and expectant management; pseudo more likely to rupture Perioperative outcome; pseudo more likely to rupture Celiac pseudo are more likely to present with rupture Case report and review Case report and review review case review review. 19

20 Tulsyan, Carr, Visceral artery Visceral artery surgical and endovascular Surveillance, open surgical repair, endovascular repair with coil embolization or glue embolization Surgical management of rupture Elective is safe and effective; urgent repair is still associated with elevated mortality rates Perioperative outcome; pseudo with increased review review tendency to rupture Recommendation 2.3 We recommend treatment of non-ruptured celiac artery true more than 2 cm in size, with a demonstrable increase in size, or with associated symptoms in patients of acceptable risk due to the risk of rupture. (Grade 1C) Stone, Vasconcelos, Graham, Tulsyan, Celiac artery Celiac artery Celiac artery aneurysm Visceral artery Surgical management, observation Open surgical Open surgical Surveillance, open surgical repair, endovascular repair No mortality noted with elective operative repair Perioperative outcome; good results with elective repair Perioperative outcome; good results with elective repair Elective is safe and effective; urgent repair is still review Case report and review case review review 20

21 Stanley, Guo, Tetreau, Visceral artery Visceral artery Visceral artery aneurysm with coil embolization or glue embolization Open surgical management Open surgical, endovascular or conservative therapy Observation and surgical or endovascular associated with elevated mortality rates Outcome of surgical management. Symptomatic patients had a higher mortality rate and re- rate. Risks of progression; good results with elective repair Review review review Recommendation 2.4 We suggest observation over for small (< 2 cm), stable asymptomatic celiac artery, or those in patients with significant medical comorbidities or limited life expectancy (Grade 2C) Stone, Corey, Celiac artery Visceral artery Surgical management, observation Observation, natural history, late outcome Late aneurysm enlargement and / or rupture were rare occurrences in treated with observation Of 73 celiac (mean initial diameter 1.6 cm) followed over a mean time of 44 months, only 5% showed evidence of enlargement review review 21

22 Tetreau, Visceral artery Surgical or endovascular management, observation Late enlargement and / or rupture were rare occurrences review 3. Treatment options Recommendation 3.1 In patients with ruptured CAA discovered at laparotomy, we suggest ligation if sufficient collateral circulation to the liver can be documented. (Grade 2C) Graham, Stone, Celiac artery Celiac artery Open surgical Abdominal imaging, open, endovascular and expectant management Successful ligation was performed in 35% of treated cases Of surgically treated cases, 11% underwent successful celiac artery ligation Outcome of Outcome of case review review Vasconcelos, Celiac artery Open surgical Case report and review Carr, Visceral artery Surgical review management of rupture Stanley, Visceral artery Open surgical Outcome of Review management Recommendation 3.2 In patients with ruptured CAA diagnosed on preoperative imaging studies who are stable, we suggest treatment with open surgical or appropriate endovascular methods based upon the patient s anatomy and underlying clinical condition. (Grade 1B) Stone, Celiac artery Abdominal imaging, open, endovascular and expectant management Outcome of review 22

23 Vasconcelos, Celiac artery Open surgical Outcome of Case report and review Ferrero, Visceral artery Open surgery, Outcome and review endovascular management complications of Shukla, Visceral artery Outcome of intact Endovascular review and ruptured visceral artery ; open surgical and endovascular for ruptured visceral has a lower morbidity and mortality than open surgical repair Cochennec, Visceral artery Open surgical and endovascular Outcome equivalent between surgical and endovascular management with regard to postoperative mortality and complications review Recommendation 3.3 For the elective treatment of CAA, we suggest using an endovascular if anatomically feasible. However, elective treatment may appropriately involve open surgical, endovascular or laparoscopic methods of depending on the patient s anatomy and underlying clinical condition. (Grade 2B) Graham, Sachdev, Celiac artery Celiac artery and superior mesenteric artery Open surgical Comparison of open surgical and endovascular therapy Outcome of Endovascular associated with decreased length of stay case review review 23

24 Zhang, Vasconcelos, Matsukura, Atkins, Kunzle, Sessa, Cochennec, Shukla, Celiac artery Celiac artery Celiac artery Celiac artery Visceral artery Visceral artery Visceral artery Visceral artery Endovascular stentgraft management Open surgical Open surgical management Endovascular with stent-graft Endovascular stentgraft management Open surgical and endovascular Open surgical and endovascular Outcome of intact and ruptured visceral artery ; open surgical and Technical success and complications. Stent-graft insertion is safe and effective Outcome of Outcome of Outcome of Early and long-term follow-up after. Stentgraft insertion is safe and effective Periprocedural outcome Outcome equivalent between surgical and endovascular management with regard to postoperative mortality and complications Outcome of Case Series Case report and review Case and review Case report review review review review 24

25 endovascular Tulsyan, Visceral artery Surveillance, open surgical repair, endovascular repair with coil embolization or glue embolization Elective is safe and effective; urgent repair is still associated with elevated mortality rates review Recommendation 3.4 To determine the need for revascularization of the celiac artery and its branches when treating CAA, we suggest evaluating the status of the superior mesenteric artery, gastroduodenal artery, and other relevant collateral circulation which must be carefully documented on preoperative imaging studies. (Grade 2B) Graham, Stone, Matsukura, Shukla, Celiac artery Celiac artery Celiac artery Visceral artery Open surgical Surgical management, observation Open surgical management Outcome of intact and ruptured visceral artery ; open surgical and endovascular Open surgical 25 Outcome of Surgical risks, outcome with observation, morbidity of rupture Outcome of Outcome of case review review Case and review review Vasconcelos, Celiac artery Outcome of Case report and review 4. Screening for concomitant Recommendation 4.1 We suggest screening patients with celiac artery for other arterial. (Grade 2B)

26 Stone, Graham, Vasconcelos, Celiac artery Celiac artery Celiac artery Surgical management, observation Open surgical Open surgical High rate of concomitant High rate of concomitant High rate of concomitant review case review Case report and review 5. Follow-up and surveillance Recommendation 5.1 In patients in whom a celiac artery aneurysm is being followed with a non-operative / nonal approach, we suggest annual surveillance with CT scans to assess for growth in size. (Grade 2B) Corey, Stone, Tetreau, Visceral artery Celiac artery Visceral artery aneurysm Natural history and outcomes after operative Abdominal imaging, open, endovascular and expectant management Observation and surgical or endovascular Of 73 celiac (mean initial diameter 1.6 cm) followed over a mean time of 44 months, only 5% showed evidence of enlargement. CT scan used for follow up of treated with observation; late enlargement was rare Relatively low risk of late enlargement or rupture. review review review 26

27 Saltzberg, Visceral artery Endovascular therapy, observation No late ruptures in patients treated with observation review Recommendation 5.2: Following endovascular for celiac artery, we suggest periodic surveillance with appropriate imaging studies to assess for the possibility of endoleak or other continued aneurysm perfusion which could lead to a continued risk of aneurysm growth or rupture. (Grade 2B) Tetreau, Sachdev, Cochennec, Carr, Sessa, Visceral artery aneurysm Celiac artery and superior mesenteric artery Visceral artery Visceral artery Visceral artery Observation and surgical or endovascular Abdominal imaging, open, endovascular and expectant management Open surgical and endovascular Open surgical and endovascular Open surgical and endovascular Early imaging following endovascular revealed a 25% rate of incomplete embolization Rate of persistent perfusion requiring re- was 11% Close imaging follow up is indicated after endovascular Perioperative and periprocedural outcome Procedural outcome. Revascularization rate following embolization was 9%. review review review review review 27

28 Gastric and Gastroepiploic Artery Aneurysms Population Interventions Outcomes Design / Quality 1. Diagnosis Recommendation 1.1: In patients who are suspected to have GA/GEA, we recommend computed tomographic angiography (CTA) as the diagnostic tool of choice (Grade 1B). Balderi, Embolization Gabelmann, Pulli, Chiaradia, patients with visceral [GA (N2)] Ruptured and nonruptured visceral (including GA x1) Ruptured and nonruptured visceral (N55) Embolization Open repair Endo 28 CTA used to confirm diagnosis in all patients, the exception being unstable patient with rupture (angiography utilized first line for appropriate ) CT performed in all patients followed by diagnostic angiography if vascular lesion identified. Review non-ruptured GEA x1 N/A N/A CTA essential to confirm the diagnosis of a ruptured visceral aneurysm and be used to plan the endovascular procedure (1) plan approach/access, (2) aneurysm morphology (procedural plan/sizing), (3) ID afferent/efferent branches, (4) determine loco-regional anatomy (collaterals) that may be relevant Review / MODERATE Review / Low

29 Recommendation 1.2: In patients suspected to have GA/GEA and have high radiation exposure risks or renal insufficiency, we recommend non-contrast MRA (NC-MRA) for diagnosis (Grade 1B). Technical remarks: NC-MRA is best suited to children and women of childbearing potential or those who have contraindications to CT or MR contrast materials (i.e. pregnancy, renal insufficiency, or gadolinium contrast material allergy). Extrapolated from renal data Recommendation 1.3: We recommend the use of catheter-based angiography for all emergent cases presenting with rupture (Grade 1B) and electively for preoperative planning (Grade 1C). Gabelmann, Ruptured and nonruptured visceral (including GA x1) 2. Criteria for invasive Embolization CT performed in all patients followed by diagnostic angiography if vascular lesion identified. Review / MODERATE Recommendation 2.1: We recommend treatment of all gastric and gastroepiploic of any size (Grade 1B). Sandstrom, Ruptured GAA Open repair Size at time of rupture provided for 1/3 of Case / LOW (9mm) Additional case reports are summarized in the published systematic review 3. Treatment options Recommendation 3.1: We recommend endovascular embolization first-line for gastric and gastroepiploic (Grade 1B). Balderi, Cochennec, Non-ruptured L gastric (N2) Non-ruptured and ruptured visceral (N40) Lt gastric aneurysm (N3) Embolization Coil Embolization (3 GAs) 0% mortality, 0% res for GA patients 0% mortality or complication for GA patients 29

30 Gabelmann, Muscari, Carmeci, Carr, Mazzaccaro, (*Can only access abstract online) Pulli, Fankhauser, Mycotic Non-ruptured 2mm GA (N1) Ruptured and nonruptured True and pseudo- visceral (GEA x2) Ruptured gastric aneurysm (N1) (31 patients with ruptured visceral in ) Ruptured right gastric pseudoaneurysm complicating pancreatitis (N1) / (26 patients with ruptured visceral in ) Ruptured and nonruptured visceral (N18) Ruptured GA aneurysm x2 Ruptured and nonruptured visceral (N55) non-ruptured GEA x1 Ruptured and nonruptured True and Embolization Embolization 0% mortality, 0% re 0% mortality, other outcomes unclear Review / MODERATE Open surgical ligation 100% mortality Open surgical ligation Open repair Open repair Endo 25% mortality for ruptures (overall); although gastric rupture survived following ligation Mortality? (3% intra-op mortality across ; 5-15% morbidity) Review Endo (majority coiling) 98% technical success; 3% early re- (within 30

31 Pseudo- (4GA and 4GEA) 30d); 3.4% aneurysm-related 30d mortality 4. Screening for concomitant Recommendation 4.1: We suggest abdominal axial imaging to screen for concomitant abdominal (Grade 2B). Inada, cases of SAM for histopathologic review (6GA, 5GEA) Open repair Multiple were found in 9 cases (33.3%) Sandstrom Tseng, Shimohira, Ruptured GAA (N3) Open repair Concurrent abdominal aneurysm (GEA) in 1/3 patients Concurrent ruptured GA and splenic aneurysm with hematemesis Splenectomy and partial gastrectomy Case / LOW No mortality Case Report / LOW 4 patients with SAM Embolization Concurrent and progressive/recurrent disease cited Case Series / LOW Recommendation 4.2: We suggest a one-time screening CTA (or MRA) of the head, neck and chest for those patients with segmental arterial mediolysis (Grade 2 C). Ro, Autopsy findings SAM with extensive arteriopathy (N1) N/A Concurrent ruptured dissecting GEA aneurysm + GA dissections + intra-cranial Autopsy Case Report / LOW Shenouda, Lit Review 62 studies of SAM considered (majority single case reports) N/A vertebral dissection (SAM) 9 cases (11%) report concomitant involvement of both abdominal and cerebral arteries Literature 5. Follow-up and surveillance Recommendation 5.1: We suggest interval surveillance (ie: every months) with axial imaging (ie: CTA or MRA) in cases of segmental medial arteriolysis in light of reported cases of rapid arterial transformation (Grade 2B). 31

32 Shimohira, patients with SAM Embolization Concurrent and progressive/recurrent disease cited Case Series / LOW Recommendation 5.2: We suggest post-embolization surveillance at every 1-2 years with axial imaging to assess for vascular remodeling and evidence of aneurysm reperfusion (Grade 2B). Bonardelli, Non-ruptured (N15) Open or video/lap-assist 0% mortality (30d) Fankhauser, Endo (majority coiling) Ruptured and nonruptured True and Pseudo- (4GA and 4GEA) Repeat imaging within the first 30 days persistent aneurysm flow was seen in five (3%) 32

33 Hepatic Artery Aneurysm Population Interventions Outcomes Design 1. Diagnosis Recommendation 1.1: In patients who are suspected to have hepatic artery aneurysm (HAA), we recommend computed tomographic angiography (CTA) as the diagnostic tool of choice (Grade 1B). Abbas, Observation and open excision, primary repair Mean Follow-up of 68.4 months case Recommendation 1.2: In patients with HAA who are considered for, we recommend mesenteric angiography for preoperative planning (Grade 1B) Abbas, Ruptured, non ruptured Observation and open excision, primary repair Mean Follow-up 68.4 months case Tulsyan, Emergent HAA Coil embolization or stent Mean Follow-up 15.6 case 2. Criteria for invasive Recommendation 2.1: Given the high propensity of rupture and significant antecedent mortality, we recommend that all hepatic artery pseudo, regardless of cause, be repaired as soon as the diagnosis is made (Grade 1A) Tuslyan, Emergent HAA Endovascular- Coil or stent All emergent cases were Frankhauser, True and False of visceral artery aneurysm Endovascularembolization or stents hepatic pseudo 80% of hepatic arteries were pseudo with 55% rate of bleeding at presentation case case Recommendation 2.2.a: We recommend repair of all symptomatic hepatic artery aneurysm regardless of size (Grade 1A). Recommendation 2.2.b: In Asymptomatic patients without significant comorbidity, we recommend repair if (true) hepatic artery aneurysm is larger than 2 cm (Grade 1A) or if aneurysm enlarges >0.5 cm/year (Grade 1C). In patients with significant comorbidities, we recommend repair if HAA is larger than 5.0 cm (Grade 1B) Abbas, Observation and open excision, primary repair Only 27% of patients with hepatic artery aneurysm of difference sizes were observed for 68 months case 33

34 Population Interventions Outcomes Design Recommendation 2.3.: We recommend repair of HAA in patients with vasculopathy or vasculitis, regardless of size (Grade 1C). Strong consideration should also be given in HAA patients with positive blood cultures (Grade 1C) Abbas, Erskine, Lal, Chan, Den Bakker, Caputo, Hassen- Khodja, Koyama, Observation and open excision, primary repair Observation and open excision and primary repair 50% of ruptured HAA cases had either fibromuscular dysplasia or polyarteritis nodosa 80% rate of rupture of HAA in patients with nonatherosclerotic Ruptured Variable Significantly higher rate of rupture in patients with nonatherosclerotic HAA 3. Treatment options Recommendation 3.1: We recommend endovascular first approach to all HAA (Grade 1A) Cochennec, Open vs. endovascular repair Sachdev, Symptomatic and asymptomatic splanchnic Symptomatic and asymptomatic Open vs. endovascular repair Similar preoperative mortality but more comorbidities among endovascular group 2.9% perioperative mortality for endovascular cases vs. 4.2% for open cases case case Case reports case case Recommendation 3.2: In patients with extrahepatic, we recommend open and endovascular techniques to maintain liver circulation (Grade 1A). 34

35 Tulsyan, Abbas, Frankhauser, Population Interventions Outcomes Design Emergent HAA Coil embolization or stent Mean Follow-up 15.6 case Observation and open Mean Follow-up of 68.4 months excision, primary repair case True and False Endovascular- 80% of hepatic arteries were of embolization or stents pseudo with 55% case visceral artery rate of bleeding at presentation aneurysm Recommendation 3.3: In patients with intrahepatic, we recommend coil embolization of the affected artery (Grade 1B). In patients with large intrahepatic artery aneurysm, we recommend resection of the involved lobe of liver to avoid significant liver necrosis (Grade 1C). Lumsden, Tulsyan, Symptomatic and asymptomatic hepatic artery Open and endovascular repair of intrahepatic Low rate of embolization of intrahepatic Study Emergent HAA Coil embolization or stent Mean Follow-up 15.6 case 4. Screening for concomitant Recommendation 4.1: We suggest abdominal axial imaging to screen for concomitant intra-abdominal in patients who did not have CTA at the time of HAA diagnosis (Grade 2B). Abbas, Observation and open excision, primary repair Mean Follow-up of 68.4 months case Recommendation 4.2: We suggest a one-time screening CTA or MRA of the head, neck and chest for those patients with non-atherosclerotic causes of hepatic artery aneurysm (Grade 2B) Lal, Chan, Den Bakker, Caputo, Ruptured Variable Significantly higher rate of rupture in patients with nonatherosclerotic HAA Case reports 35

36 Hassen- Khodja, Koyama, Erskine, Population Interventions Outcomes Design 5. Follow-up and surveillance Recommendation 5.1: We suggest annual follow-up with CTA or non-contrast CT to follow patients with asymptomatic hepatic artery aneurysm (Grade 2B). Abbas, Observation and open excision, primary repair Mean Follow-up of 68.4 months case 36

37 Superior Mesenteric Artery Aneurysms (SMAA) Population Interventions Outcomes Design 1. Diagnosis Recommendation 1.1: In patients with SMAA, we recommend computed tomographic angiography (CTA) as the diagnostic tool of choice (Grade 1B). Shanley CJ 169 Observation and open excision, primary repair CTA was more specific and sensitive than other studies Literature case Abbas MA 152 Observation and open excision, primary repair Mean Follow-up of 68.4 months case Recommendation 1.2: We recommend mesenteric angiography to delineate anatomy in preoperative planning for SMAA repair (Grade 1B) 2. Criteria for invasive Recommendation 2.1: We recommend repair of all SMA and pseudo as soon as the diagnosis is made regardless of size (Grade 1A) Shanley CJ 169 Observation and open excision, primary repair Literature case Pitton MB 94 Ruptured and nonruptured VAA Observation and open excision, coil embolization 38-50% of patients presenting with ruptured, mortality rate of case 30-90% when ruptured Kim SK 174 Non-ruptured SMAA Endovascular treatment No mortality rate with repair case Recommendation 2.2: We suggest careful observation of SMAA due to dissection unless the patient becomes symptomatic (Grade 2B) Yun WS 171 Symptomatic and asymptomatic isolated SMA dissection Observation Asymptomatic patients were remained asymptomatic (mean f/u 22 months, range of 1-80 months) case Dong Z 170 Symptomatic and asymptomatic SMA dissection Observation 37 No aneurysmal degeneration due to dissection observed with up to 48 months follow-up case

38 3. Treatment options Recommendation 3.1: We recommend endovascular first approach to all SMAA if anatomically feasible (Grade 1A) Tulsyan N 1 Emergent SMAA Coil embolization or stent Mean Follow-up 15.6; mortality rate of <5% compared to historically reported 15% for elective cases!74 case Kim SK 176 Non-ruptured SMAA Stent coverage No mortality rate with endovascular repair 4. Screening for concomitant Recommendation 4.1: We suggest abdominal axial imaging to screen for concomitant intra-abdominal in patients who did not have CTA at the time of diagnosis (Grade 2B). Tulsyan N 1 Emergent HAA Coil embolization or stent Concomitant visceral artery are common Abbas MA 152 Observation and open excision, primary repair Patients presenting with concomitant visceral artery 5. Follow-up and surveillance Recommendation 5.1: We suggest annual CTA to follow postsurgical patients (Grade 2B) Shanley CJ 169 Observation and open excision, primary repair CTA is specific and sensitive study in following these patients case case case Study 38

39 Jejunal, Ileal and Colic Artery Aneurysms Study Population Interventions Outcomes Design / Quality 1. Diagnosis Recommendation 1.1: In patients who are suspected to have JA/IA/CA, we recommend computed tomographic angiography (CTA) as the diagnostic tool of choice (Grade 1B). Balderi, Gabelmann, Pulli, Chiaradia, patients with visceral (CA x1) Ruptured and nonruptured visceral (including JA x1) Ruptured and nonruptured visceral (N55) Embolization Embolization Open repair Endo CTA used to confirm diagnosis in all patients, the exception being unstable patient with rupture (angiography utilized first line for appropriate ) CT performed in all patients followed by diagnostic angiography if vascular lesion identified. Review (CA x1) N/A N/A CTA essential to confirm the diagnosis of a ruptured visceral aneurysm and be used to plan the endovascular procedure (1) plan approach/access, (2) aneurysm morphology (procedural plan/sizing), (3) ID afferent/efferent branches, (4) determine loco-regional Review / MODERATE Review / Low 39

40 Study Population Interventions Outcomes Design / Quality anatomy (collaterals) that may be relevant Recommendation 1.2: In patients with high radiation exposure risks or renal insufficiency, we recommend non-contrast MRA (NC-MRA) for diagnosis (Grade 1B). Technical remarks: NC-MRA is best suited to children and women of childbearing potential or those who have contraindications to CT or MR contrast materials (i.e. pregnancy, renal insufficiency, or gadolinium contrast material allergy). Extrapolated from renal data Recommendation 1.3: We recommend the use of catheter-based angiography for all emergent cases presenting with rupture (Grade 1B) and electively for preoperative planning (Grade 1C). Gabelmann, Ruptured and nonruptured visceral Embolization CT performed in all patients followed by diagnostic angiography if vascular lesion identified. Review / MODERATE Recommendation 1.4: We suggest screening all patients with JA, IA and CA for vasculitis with routine inflammatory markers (Grade 2C). Hong, Ruptured ileocolic artery aneurysm Embolization (and medical management) Ruptured ileocolic aneurysm secondary to Bechet s Case report / LOW Sellke, Ruptured middle colic artery Open and endovascular Supports 4 cases of ruptured ilio- and colic Tessier, Ruptured and nonruptured branch (JA x5, IA x2, CA x4) 2. Criteria for invasive Embolization Ligation Colectomy Conservative resulting from PAN -All ruptured were colic (1cm or unknown in size) - 1/3 ruptured aneuryusms had case of PAN Case report and Lit review / LOW Recommendation 2.1 We recommend elective for jejunal and ileal >2cm in maximal diameter and for all colic, any size (Grade 1B). 40